Stauffer syndrome in a tumor of the pancreatic tail: A paracrine pathogenesis, rather than an inflammatory disimmune phenomenon – a case report

Abstract

Stauffer syndrome, also known as paraneoplastic intrahepatic cholestasis syndrome, is a rare reversible clinical manifestation characterized by elevation of direct bilirubin, alcaline phosphatase, transaminases and prolonged prothrombin time, without direct hepatobiliary disease, that is due to the presence of malignancy, mostly kidney cancer. In this case report, we describe a rare form of mixed acinar neuroendocrine pancreatic tail mass causing a non-obstructive form of cholestasis. We analysed the effects of metilprednisolone treatment and surgery on the levels of interleukin-6 (IL-6) and tumor necrosis factor alpha (TNF alpha), previously associated with this syndrome. We found that serum levels of IL-6 and TNF alpha decreased during steroid treatment, had a peak immediately after the surgical procedure, and remained stable while cholestasis resolved slowly after tumour removal. These findings showed that IL-6 and TNF alpha were not the effectors of the paraneoplastic cholestasis in our patient. Rather, the evidence of a mixed acinar neuroendocrine pancreatic tumour and non-specific cholestasis at the liver histology suggests paracrine mechanisms affecting cellular transporters implicated in bile acids excretion.

Keywords

• Stauffer syndrome
• Paraneoplastic cholestasis
• Cytokine
• Pancreatic neuroendocrine tumour (NET)
• Non obstructive cholestasis
• Case report